Current management and outcome of pregnancies in women with adrenal insufficiency: experience from a multi-center survey

Context: Appropriate management of adrenal insufficiency (AI) in pregnancy can be challenging due to the rarity of the disease and lack of evidence-based recommendations to guide glucocorticoid- and mineralocorticoid dosage adjustment. Objective: Multi-center survey on current clinical approaches in managing AI during pregnancy. Design: Retrospective anonymized data collection from 19 international centers from 2013-2019. Setting and patients: 128 pregnancies in 113 women with different causes of AI: Addison disease (44%), secondary AI (25%), congenital adrenal hyperplasia (25%) and acquired AI due to bilateral adrenalectomy (6%). Results: Hydrocortisone (HC) was the most commonly used glucocorticoid in 82.9% (97/117) of pregnancies. Glucocorticoid dosage was increased at any time during pregnancy in 73/128 (57%) of cases. In these cases, difference of the daily dose of HC equivalent between baseline and the third trimester was 8.6±5.4 [range: 1, 30] mg. Fludrocortisone dosage was increased in fewer cases (7/54 during the first trimester, 9/64 during the second trimester and 9/62 cases during the last trimester). Overall, an adrenal crisis was reported in 9/128 (7%) pregnancies. Caesarian section was the most frequent mode of delivery at 58% (69/118). Fetal complications were reported in 3/120 (2.5%) and minor maternal complications in 15/120 (12.5%) pregnancies without fatal outcomes. Conclusions: This survey confirms good maternal and fetal outcome in women with AI managed in specialized endocrine centers. An emphasis on careful endocrine follow up and repeated patient education is likely to have reduced the risk of adrenal crisis and resulted in positive outcomes.